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Sirolimus is used to treat GVHD (graft versus host disease) and is not coded as treatment. Even though the sub-category is mTOR inhibitor it does not automatically mean it is a chemotherapeutic agent. Sirolimus affects cells differently than Temsirolimus.  The chemical compounds differ between these drugs. In order to code rapamycin, the drug given must be stated to be either the analog or ester compound.  The SEER*RX database has been corrected and NSCLC/glioblastoma are no longer listed for sirolimus.

 We researched clinical trials and found several that include sirolimus with other chemotherapy drugs for patients who either have received or will be receiving bone marrow transplants for hematologic diseases. In this case it is not coded. There are a few trials that are looking at sirolimus as a treatment for bladder, prostate, nerve sheath tumors, MDS, and AML. For these cases it would be coded in Other (code 2).

Sites may be added to certain combinations when indicated by ClinCORE review for Cancer PathCHART. Please note some sites were added in the 2024 update as a result of PathCHART review. A newly-formed Solid Tumor Editorial Board and its subgroups are currently working to evaluate the Solid Tumor Manual and make recommendations on ways to improve the structure and formatting of the manual and its content.

Follow the rules and instructions in the Other Sites STRs when assigning combination histology codes.

Histology Coding Rules

Use the Histology Coding Rules when assigning combination codes.

Coding Histology Information

Use this section that includes the mixed histology (Table 2) and site-specific histology tables (Tables 3-23) for one or more histologies within a single tumor. Do not use this section in place of the Histology Coding Rules.

While site-specific histology tables, based on current WHO Classification of Tumors books, have been added to Other Sites STRs, not all site groups have individual histology tables; coding may require the use of ICD-O and updates. The histology tables in Other Sites STRs include additional coding instructions and notes to assign the correct ICD-O code when appropriate.

The tables are not meant to be all-inclusive; rather they are intended to address difficult coding situations to facilitate the assignment of the correct histology code.

Table 2: Mixed and Combination Codes Instructions

Once you have identified the histology terms and have been instructed to use Table 2 by the Histology Coding Rules, compare the terms in the diagnosis to the terms in Column 1. When the terms match, use the combination code listed in Column 2. Use adenocarcinoma mixed subtypes 8255 as a “last resort” code.

Smoldering systemic mastocytosis is reportable, 9741/3. Indolent systemic mastocytosis is not reportable as of cases diagnosed 2018, 9741/1.

Smoldering systemic mastocytosis and indolent systemic mastocytosis are not synonymous. Smoldering differs from indolent based on diagnostic criteria and burden of disease; indolent is low whereas smoldering is high burden of disease that can progress to aggressive systemic mastocytosis or mast cell leukemia.

We will update SINQ 20130134.

Solid Tumor rule H6 is the same as MP/H rule H8. We found registrars have problems understanding reportable terminology and the corresponding ICD-O-3 histology code for lentigo maligna melanoma. It is included in H6 to capture cases where the registrar may not stop at H1. We will add another note to H1 instructing users to continue through the rules if the diagnosis is lentigo maligna melanoma.

Solid pseudopapillary neoplasm of the pancreas is reportable for cases diagnosed in 2014 and later. Report solid pseudopapillary neoplasm of the pancreas (8452/3) as the guidance in SINQ 20140058 is still in effect.

The 4th and 5th editions of the WHO Classification of Tumors of the digestive system define solid pseudopapillary neoplasm of the pancreas as a low-grade malignant pancreatic tumor.

Solid pseudopapillary neoplasm of the pancreas is reportable. According to the WHO classification, it is a "low-grade malignant neoplasm…[which] frequently undergoes hemorrhagic-cystic degeneration and occurs predominantly in young women."

Assign topography code C25 with the appropriate 4th digit. Code the histology as 8452/3.

Spinal cord tumors (including lipomas) are reportable when they arise in the spinal dura or nerve root. The tumor must be of the spinal cord itself or within the spinal cord dura. Spinal cord tumors are reportable when they arise in the intradural space. A reportable intradural tumor can be either intramedullary or extramedullary. Extramedullary intradural spinal tumors are reportable. A spinal tumor originating in the extradural space is not reportable. If it is outside the dura, it is not reportable because it would be outside the CNS. They are not reportable when they arise in the peripheral nerves.

Squamous carcinoma and squamous intraepithelial neoplasia III arising in the skin of the perineum (C445) are not reportable. Even though the abreviation "VIN III" is used in this example, this lesion does not involve the vulva. Since it involves the perineum, and skin of perineum is coded to C445, it is not reportable.

Neoplasms arising in skin (C44) with the following histologies are not reportable.

--Malignant neoplasm (8000-8005)

--Epithelial carcinoma (8010-8046)

--Papillary and squamous cell carcinoma (8050-8084)

--Squamous intraepithelial neoplasia III (8077) arising in perianal skin (C445)

--Basal cell carcinoma (8090-8110)

Squamous mucosa with high grade dysplasia is the same as high grade squamous dysplasia in the conjunctiva and is coded to 8077/2.

Stage this case the same as all other cases. Use only the information subsequent to the date of diagnosis to code stage and treatment.

The diagnosis date in the example is 04/2003. Do not use information prior to 04/2003 to code stage or treatment. Do not code the preventative chemo as treatment.